General Patton- Bullous and Melanocytic Dz Flashcards
Which subtype of melanoma is most likely to be present for a long time before being diagnosed?
Lentigo maligna melanoma
Involvement of what structues concurrent with congenital nevi carries a poor prognosis (regardless of malignant transformation)?
leptomeninges (pia and arachnoid)
What is the big issue with mucous membrane pemphigoid?
It results in scarring at whatever mucosal sites it involves (corneal blindness, esophageal stenosis, laryngeal stenosis)
Name 2 genes that have mutations which predispose to melanoma
CDKN2A and BRAF
What are vemurafinib and dabrafenib?
BRAF inhibitors for Tx of melanoma
What is the most common immunobullous disease?
bullous pemphigoid
What is the most common subtype of melanoma?
superficial spreading
MOA of ipilimumab
binds CTLA4 to disinhibit T cells for Tx of melanoma
What is the Tx of Linear IgA disease when not caused by an underlying drug problem?
Dapsone
What is the DOC for dermatitis herpetiformis?
dapsone but for long-term they need the gluten free diet
What drug binds CTLA-4
Ipilimumab
What is the most common malignancy associated with paraneoplastic pemphigus?
Non-Hodgkin’s lymphoma
Autoantibodies to what kind of structures are present in the pemphigus group of immunobullous diseases?
desmosomes
How is the Tx of mucous membrane pemphigoid different from that of bullous pemphigoid and pemphigus vulgaris?
Mucous membrane pemphigoid involves more rigorous tx not only with corticosteroids but also cyclophosphamide because of the serious sequela that can occur
T/F: most patients with dermatitis hepetiformis have celiac sprue
False? ALL patients with dermatitis herpetiformis have celiac sprue
What must you work up a patient for if they have porphyria cutanea tarda?
liver disease
What immunobullous disease is treated with dapsone?
Linear IgA disease that is NOT associated with drugs, if it is then the Tx is to remove the drug; dermatitis herpetiformis
What are the autoantibodies against in pemphigus vulgaris? Bullous pemphigoid?
Pemphigus vulgaris = desmoglein of desmosomes; bullous pemphigoid = BPAg1 and 2
What is the most common cause of Linear IgA disease?
Vancomycin and other drugs
What dermatologic disease can rituximab (anti-CD21) be used to Tx?
Pemphigus vulgaris
What is the Tx of Linear IgA disease?
Removal of the offending agent
What is a nevus that is present at birth?
Congenital nevus. OMG brain-usage
What are the autoantibodies targeting in bullous pemphigoid?
BPAg 1 and 2 (Bullous pemphigoid antigen 1 and 2)
Which type of melanoma has the highest rate of metastasis?
Nodular melanoma (because it grows down into skin and staging is by depth of invasion)
What are the steroid-sparing therapies for pemphigus vulgaris?
mycophenolate mofetil or azathioprine (and rituximab)
How does Dr. Hussein pronounce “occur”?
Oak-hair
What is a nevus that occurs in childhood or adulthood called?
A benign acquired nevus
Compare and contrast the Tx of bullous pemphigoid and pemphigus vulgaris
They are actually the same? First use corticosteroids and follow up with steroid sparing drugs (mycophenolate moxetil and azathioprine) once under control. BUT rituximab seems to be reserved only for pemphigus vulgaris
What demographic experiences the highest mortality rates with melanoma?
older men
Which type of melanoma contains no pigmentation?
amelanotic
What is the autoantigen in epidermolysis bullosa acquisita?
Type VII collagen
What are the 3 diseases under the pemphigus grouping?
Pemphigus vulgaris, Pemphigus foliaceous, and Paraneoplastic pemphigus
T/F: nuclear atypia in a dysplastic nevus is associated with a clinically benign outcome
True, dysplastic nevi are treated as benign conditions
Where do melanomas most often arise?
In areas of skin where a nevus was not previously present
Which porphyria can be acquired with liver failure and HIV?
porphyria cutanea tarda
Name the 2 BRAF inhibitors for melanoma
vemurafinib and dabrafenib
What is the best way to remove a suspicious looking mole?
elliptical excision (as opposed to shave, saucerization, or punch)
What clinical sign can dermatologists use to tell which of a group of nevi is most likely to be melanoma
The Ugly Duckling sign–and these people need the highest board scores of all
MOA of trametinib
Inhibits MEK when treating Melanoam
Where are the lesions in pemphigus vulgaris?
In the mouth and skin? Bullous pemphigoid really does not involve the mouth unless it is just mucous membrane pemphigoid
T/F: sentinal lymph node biopsy is a therapeutic procedure for mgmt of melanoma
False, it is done to obtain prognostic information
What color are Spitz nevi?
pink to red
Enzyme deficient in porphyria cutanea tarda
uroporphyrinogen decarboxylase; note, in case of distractor that acute intermittent porphyria is urobilinogen deaminase
When Txing melanoma, what drug inhibits MEK?
Trametinib
This disease involves autoantibodies against type VII collagen
Epidermolysis bullosa acquisita NOT the hereditary epidermolysis bullosa
How is pemphigus foliaceous different from pemphigus vulgaris?
It is not as severe and they do NOT develop oral lesions
Which type of melanoma occurs mostly in African American, Africans, and Asians?
Acral lentiginous melanoma
When is there a risk of melanoma in congenital nevi?
If they are very large
How do you tx porphyria cutanea tarda?
phlebotomy, the underlying cause is excess iron i.e. alcoholic cirrhosis, hemochromatosis and hepatocellular carcinoma? also LOW DOSE CHLOROQUINE
What is the mainstay of Tx for pemphigus vulgaris?
Corticosteroids
After removal of a melanoma, what dictates the next step in its management?
The thickness of the original tumor
How is the oral disease of paraneoplastic pemphius different from pemphigus vulgaris and foliaceous?
It is the most severe and pemphigus foliaceous doesn?t have oral involvement anyway
If melanoma occurs in a congenital nevus, when is the earliest that is likely to occur?
puberty
T/F: one can make the diagnosis of dysplastic nevus clinically
nope, dysplastic nevus is a histological Dx
What are the autoantibodies targeting in pemphigus vulgaris?
desmoglein of desmosomes
